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Zebrafish Motile Cilia as a Model for Primary Ciliary Dyskinesia

Zebrafish is a vertebrate teleost widely used in many areas of research. As embryos, they develop quickly and provide unique opportunities for research studies owing to their transparency for at least 48 h post fertilization. Zebrafish have many ciliated organs that include primary cilia as well as...

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Autores principales: Pinto, Andreia L., Rasteiro, Margarida, Bota, Catarina, Pestana, Sara, Sampaio, Pedro, Hogg, Claire, Burgoyne, Thomas, Lopes, Susana S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8393663/
https://www.ncbi.nlm.nih.gov/pubmed/34445067
http://dx.doi.org/10.3390/ijms22168361
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author Pinto, Andreia L.
Rasteiro, Margarida
Bota, Catarina
Pestana, Sara
Sampaio, Pedro
Hogg, Claire
Burgoyne, Thomas
Lopes, Susana S.
author_facet Pinto, Andreia L.
Rasteiro, Margarida
Bota, Catarina
Pestana, Sara
Sampaio, Pedro
Hogg, Claire
Burgoyne, Thomas
Lopes, Susana S.
author_sort Pinto, Andreia L.
collection PubMed
description Zebrafish is a vertebrate teleost widely used in many areas of research. As embryos, they develop quickly and provide unique opportunities for research studies owing to their transparency for at least 48 h post fertilization. Zebrafish have many ciliated organs that include primary cilia as well as motile cilia. Using zebrafish as an animal model helps to better understand human diseases such as Primary Ciliary Dyskinesia (PCD), an autosomal recessive disorder that affects cilia motility, currently associated with more than 50 genes. The aim of this study was to validate zebrafish motile cilia, both in mono and multiciliated cells, as organelles for PCD research. For this purpose, we obtained systematic high-resolution data in both the olfactory pit (OP) and the left–right organizer (LRO), a superficial organ and a deep organ embedded in the tail of the embryo, respectively. For the analysis of their axonemal ciliary structure, we used conventional transmission electron microscopy (TEM) and electron tomography (ET). We characterised the wild-type OP cilia and showed, for the first time in zebrafish, the presence of motile cilia (9 + 2) in the periphery of the pit and the presence of immotile cilia (still 9 + 2), with absent outer dynein arms, in the centre of the pit. In addition, we reported that a central pair of microtubules in the LRO motile cilia is common in zebrafish, contrary to mouse embryos, but it is not observed in all LRO cilia from the same embryo. We further showed that the outer dynein arms of the microtubular doublet of both the OP and LRO cilia are structurally similar in dimensions to the human respiratory cilia at the resolution of TEM and ET. We conclude that zebrafish is a good model organism for PCD research but investigators need to be aware of the specific physical differences to correctly interpret their results.
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spelling pubmed-83936632021-08-28 Zebrafish Motile Cilia as a Model for Primary Ciliary Dyskinesia Pinto, Andreia L. Rasteiro, Margarida Bota, Catarina Pestana, Sara Sampaio, Pedro Hogg, Claire Burgoyne, Thomas Lopes, Susana S. Int J Mol Sci Article Zebrafish is a vertebrate teleost widely used in many areas of research. As embryos, they develop quickly and provide unique opportunities for research studies owing to their transparency for at least 48 h post fertilization. Zebrafish have many ciliated organs that include primary cilia as well as motile cilia. Using zebrafish as an animal model helps to better understand human diseases such as Primary Ciliary Dyskinesia (PCD), an autosomal recessive disorder that affects cilia motility, currently associated with more than 50 genes. The aim of this study was to validate zebrafish motile cilia, both in mono and multiciliated cells, as organelles for PCD research. For this purpose, we obtained systematic high-resolution data in both the olfactory pit (OP) and the left–right organizer (LRO), a superficial organ and a deep organ embedded in the tail of the embryo, respectively. For the analysis of their axonemal ciliary structure, we used conventional transmission electron microscopy (TEM) and electron tomography (ET). We characterised the wild-type OP cilia and showed, for the first time in zebrafish, the presence of motile cilia (9 + 2) in the periphery of the pit and the presence of immotile cilia (still 9 + 2), with absent outer dynein arms, in the centre of the pit. In addition, we reported that a central pair of microtubules in the LRO motile cilia is common in zebrafish, contrary to mouse embryos, but it is not observed in all LRO cilia from the same embryo. We further showed that the outer dynein arms of the microtubular doublet of both the OP and LRO cilia are structurally similar in dimensions to the human respiratory cilia at the resolution of TEM and ET. We conclude that zebrafish is a good model organism for PCD research but investigators need to be aware of the specific physical differences to correctly interpret their results. MDPI 2021-08-04 /pmc/articles/PMC8393663/ /pubmed/34445067 http://dx.doi.org/10.3390/ijms22168361 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Pinto, Andreia L.
Rasteiro, Margarida
Bota, Catarina
Pestana, Sara
Sampaio, Pedro
Hogg, Claire
Burgoyne, Thomas
Lopes, Susana S.
Zebrafish Motile Cilia as a Model for Primary Ciliary Dyskinesia
title Zebrafish Motile Cilia as a Model for Primary Ciliary Dyskinesia
title_full Zebrafish Motile Cilia as a Model for Primary Ciliary Dyskinesia
title_fullStr Zebrafish Motile Cilia as a Model for Primary Ciliary Dyskinesia
title_full_unstemmed Zebrafish Motile Cilia as a Model for Primary Ciliary Dyskinesia
title_short Zebrafish Motile Cilia as a Model for Primary Ciliary Dyskinesia
title_sort zebrafish motile cilia as a model for primary ciliary dyskinesia
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8393663/
https://www.ncbi.nlm.nih.gov/pubmed/34445067
http://dx.doi.org/10.3390/ijms22168361
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